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A syndrome characterized by a unique combination of cardiac arrhythmias and intestinal pseudo-obstruction. It has material basis in the mutated SGOL1 protein. Distinctive clinical features include atrial dysrhythmias, sick sinus syndrome (SSS) and valve anomalies and chronic intestinal pseudo-obstruction (CIPO).
Features include always present findings: Decreased body weight, Intestinal pseudo-obstruction, Bradycardia, and Sick sinus syndrome and others; and common findings: Left atrial enlargement. 12 total HPO annotations.
Organ System | Phenotype Count | Example Features |
|---|---|---|
Heart and blood vessels | 7 | Bradycardia, Ventricular escape rhythm, Atrial fibrillation |
SGO1 function has not been fully characterized.
Chronic atrial and intestinal dysrhythmia is associated with mutations in the SGO1 gene on chromosome 3.
Genetic testing for SGO1 is available. Testing is considered confirmatory for diagnosis.
Phenotype severity distribution: 5 always present features, 1 common feature.
Estimated prevalence: <1 in 1,000,000 (VERY_RARE).
No clinical trials have been registered for chronic atrial and intestinal dysrhythmia.
10 publications have been identified in PubMed for chronic atrial and intestinal dysrhythmia. Research spans Basic Science / Preclinical (44%), Case Report / Case Series (33%), and Other (11%).
Hosseini A (2026). [PMID: 41767060](https://pubmed.ncbi.nlm.nih.gov/41767060/). *Clin Case Rep*. [Case Report / Case Series]
Chen W (2026). [PMID: 41380984](https://pubmed.ncbi.nlm.nih.gov/41380984/). *Biochim Biophys Acta Gen Subj*. [Basic Science / Preclinical]
Pan F (2025). [PMID: 40788381](https://pubmed.ncbi.nlm.nih.gov/40788381/). *Mol Biol Rep*. [Basic Science / Preclinical]
Padilha SF (2025). [PMID: 40258920](https://pubmed.ncbi.nlm.nih.gov/40258920/). *Sci Rep*. [Basic Science / Preclinical]
Ahuja K (2025). [PMID: 40386336](https://pubmed.ncbi.nlm.nih.gov/40386336/). *JPGN Rep*. [Case Report / Case Series]
Data assembled from 6 of 12 sources · Last updated Sep 19, 2026, 2:28 AM UTC
Online Mendelian Inheritance in Man
European rare disease database
Genetic and Rare Diseases Info Center
Common questions about chronic atrial and intestinal dysrhythmia
Digestive system |
1 |
Intestinal pseudo-obstruction |
Growth and development | 1 | Failure to thrive |
Thomas HR (2025). [PMID: 40475461](https://pubmed.ncbi.nlm.nih.gov/40475461/). *bioRxiv*. [Basic Science / Preclinical]
Smit RAJ (2025). [PMID: 40691366](https://pubmed.ncbi.nlm.nih.gov/40691366/). *Nat Med*. [Epidemiology / Natural History]
Zengin O (2024). [PMID: 39733308](https://pubmed.ncbi.nlm.nih.gov/39733308/). *J Gastrointestin Liver Dis*. [Other]
AI-curated news mentioning chronic atrial and intestinal dysrhythmia
Updated May 7, 2026
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A position paper from the Society for Paediatric Gastroenterology and Nutrition discusses the use of teduglutide in treating short bowel syndrome in infants, children, and adolescents. This guidance aims to inform clinical practices regarding chronic intestinal failure management.